Malignant peripheral nerve sheath tumor
Alerts and Notices
Synopsis
Neurofibromas may transform into MPNSTs. This is of specific concern in patients with neurofibromatosis, who may have a high number of peripheral nerve tumors.
MPNSTs have a poor prognosis generally, with a worse prognosis in people with large tumors or neurofibromatosis. The primary treatment is surgical resection.
Codes
ICD10CM:C47.9 – Malignant neoplasm of peripheral nerves and autonomic nervous system, unspecified
SNOMEDCT:
404037002 – Malignant peripheral nerve sheath tumor
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Neurofibroma
- Schwannoma
- Perineurioma
- Ganglioneuroma
- Metastatic cancer
- Neurolymphomatosis
- Local hematoma with nerve compression
- Neuroma
- Trauma (see traumatic neuroma)
- Vasculitis
- Nerve infarction
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Management Pearls
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Therapy
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References
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Last Reviewed:04/04/2019
Last Updated:04/22/2019
Last Updated:04/22/2019
Malignant peripheral nerve sheath tumor